Hepatology 1994 Jul;20(1 Pt 2):41S-48S

Sequential treatment of biliary atresia with Kasai portoenterostomy and live
transplantation: a review

Otte JB, de Ville de Goyet J, Reding R, Hausleithner V, Sokal E, Chardot C,
Debande B
Department of Pediatric Surgery, Cliniques Universitaires Saint-Luc, Brussels,
Belgium

Abstract
Biliary atresia is the most frequent cause of chronic cholestasis in infants.
When left untreated, this condition leads to death from liver insufficiency
within the first 2 yr of life. The modern therapeutic approach consists of a
sequential strategy with Kasai portoenterostomy as a first step and, in case of
failure, liver transplantation. After portoenterostomy, no more than 20% to 30%
of patients will live jaundice-free into adulthood. Illness in another third
will be palliated, and these patients have extended survival, delaying liver
transplantation to later childhood (2 to 15 yr). The remaining 30% to 40% will
not benefit from the Kasai operation and will die of liver failure in infancy.
The annual need of liver transplantation for biliary atresia is one case per
million people. This indication represents 35% to 67% of the reported series of
pediatric liver transplantation and between 5% and 10% of the indications for
liver transplantation, all ages included. Approximately four of five children
transplanted for biliary atresia will become long-term survivors with good
physical and mental development; recurrence of the disease after transplantation
has not been observed. Because most candidates are young children (< 3 yr) of
small size (< 10 kg), there is a shortage of size-matched donors (which has been
alleviated by the use of innovative techniques such as reduced and split
livers). The resulting redistribution of the adult donor liver pool is ethically
justified by the equal quality of the results after transplantation of a
full-size or partial graft